Scleroderma

Question 1

Scleroderma is an

Answer 1

inflammatory disease
involves vascular sclerosis
fibrosis of skin & viscera
injury to vascular endothelium resulting in leakage of proteins into interstitial space
collagen production is not slowed down & gets deposited throughout the body

Question 2

Scleroderma is considered to be a

Answer 2

collagen vascular disease with autoimmune characterstics

Question 3

Scleroderma typically affects

Answer 3

women
20-40 years old at onset
accelerated by pregnancy

Question 4

The etiology of scleroderma is

Answer 4

unknown

Question 5

CREST syndrome is

Answer 5

Calcinosis- Ca2+ deposits
Raynaud's phenomenon
Esophageal hypomobility
Sclerodactyly- thickened/tight skin
telangiectasia- dilated capillaries leading to red marks on skin

Question 6

Describe the key difference between diffuse and limited scleroderma.

Answer 6

diffuse- short interval (<1 year) between the onset of Raynaud’s phenomenon and the development of skin changes
limited- long history of Raynaud’s phenomenon

Question 7

Skin & musculoskeletal effects of scleroderma include

Answer 7

skin is thickened
diffuse edema
contractures
skeletal muscle myopathy 
arthritis & limited joint mobility
avascular necrosis

Question 8

Describe how scleroderma affects the nervous system.

Answer 8

peripheral and cranial neuropathies due to nerve compression by thickened connective tissue
-trigeminal neuralgia–> producing lots of pain
dry eyes

Question 9

Describe how scleroderma affects the cardiovascular system.

Answer 9

sclerosis of coronary arteries and conduction system
replacement of cardiac tissue with fibrous tissue
systemic and pulmonary HTN
pericarditis and pericardial effusion
peripherally- intermittent vasospasm
Raynaud’s phenomenon

Question 10

Describe how scleroderma affects the respiratory system.

Answer 10

Diffuse interstitial pulmonary fibrosis
arterial hypoxemia secondary to decreased diffusion capacity
decreased pulmonary compliance

Question 11

Describe how scleroderma affects the kidneys.

Answer 11

causes renal artery stenosis due to arteriolar intimal proliferation
decreased renal blood flow
hypertension

Question 12

Describe how scleroderma affects the GI system.

Answer 12

Dry oral mucosa
progressive fibrosis of GI tract
dysphagia
hypomotility
decreased lower esophageal sphincter tone–> increased aspiration risk
malabsorption–> vitamin K deficiency leading to coagulopathies (2, 7, 9 & 10)

Question 13

Describe anesthesia concerns for patients with scleroderma.

Answer 13

protect eyes from corneal abrasion
dermal thickening leads to difficult IV access
fibrosis may lead to limited mouth opening and difficult intubation
hypotonia of LES makes them an aspiration risk
regional anesthesia may be challenging with contractures & decreased joint mobility

Question 14

Describe the anesthetic considerations related to the respiratory system for the scleroderma patient.

Answer 14

pulmonary hypertension–> vent & perfusion concerns
decreased pulmonary compliance and decreased O2 diffusion
sensitive to respiratory depressants

Question 15

Describe the anesthetic considerations related to the renal system for the scleroderma patient.

Answer 15

renal dysfunction and drug elimination

Question 16

Describe the anesthetic considerations related to the CV system for the scleroderma patient.

Answer 16

chronic systemic HTN may have a contracted intravascular volume